Retinitis pigmentosa is an
eye disorder and is the name given to a group of hereditary diseases
of the retina - the light sensitive tissue in the back of the eye. The
retina slowly degenerates and gradually loses its ability to transmit
images to the brain, resulting in a progressive loss in
vision. Cells from
the pigmented layer of the retina migrate into the nerve cell containing
layer, causing a typical pattern of black or brown star shapes in the
retina that give the diseases their name.
Age of onset and worsening
Symptoms of retinitis pigmentosa usually become apparent between the ages
of ten and 30. Patients typically lose night
vision in adolescence, side vision in young adulthood, and central vision in
later life because of progressive loss of rod and cone photoreceptor cells.
Yearly evaluation of the progression of the disease is recommended.
Retinitis Pigmentosa treatment with supplements
Fish oils supplements or
DHA could be useful,
along with lutein
carotenoid.
Invest Ophthalmol Vis Sci. 2015. Docosahexaenoic Acid Slows Visual Field Progression in X-Linked Retinitis Pigmentosa: Ancillary Outcomes of the DHAX Trial. Docosahexaenoic acid (DHA) was supplemented in a single-site, placebo-controlled, randomized clinical trial designed to slow vision loss associated with X-linked retinitis pigmentosa (XLRP); the DHAX Trial. Male participants with XLRP (range, 7-31 years) received 30 mg DHA/kg/d (n = 29) or placebo (n = 22) for 4 years. Visual outcomes were measured annually and red blood cell (RBC) DHA determined every 6 months. Oral DHA supplementation increased mean RBC-DHA levels by 4-fold over placebo. No group differences in progression were found for visual acuity, shape discrimination, or fundus appearance. Yearly rates of progression were reduced for dark-adapted thresholds and visual field sensitivity for foveal, macular, peripheral, total, and ellipsoid zone regions by DHA supplementation. Supplementation of DHA significantly elevated blood DHA levels and reduced the rate of progression in final dark-adapted thresholds and visual field sensitivity. From the relationship between RBC-DHA and the rate of field sensitivity loss, we can extrapolate that an RBC-DHA level of 17% could minimize the decline in field sensitivity.
Chlorogenic acid
J Korean Med Sci. January 2014. Chlorogenic Acid supplementation improves
multifocal electroretinography in patients with retinitis pigmentosa. To
evaluate the effect of chlorogenic acid supplementation in patients with
retinitis pigmentosa, we evaluated objective change in visual function with
multifocal electroretinography, along with visual acuity, visual field, standard
electroretinography, and contrast sensitivity. Eighteen patients diagnosed with
retinitis pigmentosa were enrolled in this prospective, non-comparative,
single-arm study. Multifocal electroretinography, best-corrected visual acuity
in Early Treatment Diabetic Retinopathy Study letters, total point score on
visual field examination with Humphrey Field Analyzer II, electroretinography,
and contrast sensitivity were measured and repeated after 3 months
supplementation with chlorogenic acid. The amplitude of ring 5 was significantly
higher on multifocal electroretinography after 3 months of chlorogenic acid
supplementation. There were no significant changes in the best-corrected visual
acuity, total point score on Humphrey Field Analyzer, 30 Hz flicker amplitude on
standard electroretinography, or contrast sensitivity. Chlorogenic acid may have
a beneficial effect on the peripheral area at the margins of retinal
degeneration, and should be considered as an anti-oxidant for the management of
retinitis pigmentosa.
Lutein
Clinical trial of lutein
in patients with retinitis pigmentosa receiving
vitamin A.
Arch Ophthalmol. 2010. Berman-Gund Laboratory
for Study of Retinal Degenerations, Harvard Medical School,
Massachusetts Eye and Ear Infirmary, Boston, MA, USA.
Randomized, controlled, double-masked trial of 225 nonsmoking patients,
aged 18 to 60 years, evaluated over a 4-year interval. Patients received
12 mg of lutein or a control tablet daily. All were given 15,000 IU/d of
vitamin A palmitate. Randomization took into account genetic type and
baseline serum lutein level. The primary outcome was the total point
score for the Humphrey Field Analyzer (HFA) 30-2 program; prespecified
secondary outcomes were the total point scores for the 60-4 program and
for the 30-2 and 60-4 programs combined, 30-Hz electroretinogram
amplitude, and Early Treatment Diabetic Retinopathy Study acuity. No
significant difference in rate of decline was found between the lutein
plus vitamin A and control plus vitamin A groups over a 4-year interval
for the HFA 30-2 program. For the HFA 60-4 program, a decrease in mean
rate of sensitivity loss was observed in the lutein plus vitamin A
group. Mean decline with the 60-4 program was slower among those with
the highest serum lutein level or with the highest increase in macular
pigment optical density at follow-up. Those with the highest increase in
macular pigment optical density also had the slowest decline in HFA 30-2
and 60-4 combined field sensitivity. No significant toxic effects of
lutein supplementation were observed. Lutein supplementation of 12
mg/d slowed loss of midperipheral visual field on average among
nonsmoking adults with retinitis pigmentosa taking vitamin A.
Application to Clinical Practice Data are presented that support use of
12 mg/d of lutein to slow visual field loss among nonsmoking adults with
retinitis pigmentosa taking vitamin A.
Lutein supplementation in retinitis pigmentosa: PC-based vision assessment in a
randomized double-masked placebo-controlled clinical trial.
BMC Ophthalmol. 2006. Bahrami H, Melia M, Dagnelie G. The Wilmer
Eye Institute, Department of Ophthalmology, School of Medicine, Johns Hopkins
University, Baltimore, MD, USA.
There is no generally accepted medical or surgical treatment to stop the
progressive course of retinitis pigmentosa. Previous studies have suggested
lutein as a potential treatment with positive effects on macular pigment
density. The objective of this study was to examine the effect of lutein
supplementation on preservation of visual function in patients with retinitis
pigmentosa. In a double-masked randomized placebo-controlled phase I/II clinical
trial with a cross-over design, 34 adult patients with retinitis pigmentosa were
randomized to two groups. One group, consisted of 16 participants, received
lutein supplementation (10 mg/d for 12 wks followed by 30 mg/d) for the first 24
weeks and then placebo for the following 24 weeks, while the other group
included 18 participants for whom placebo (24 weeks) was administered prior to
lutein. For visual acuity (VA) at normal illumination level, treatment with
lutein reduced logMAR, i.e. improved VA, but this effect was not statistically
significant. Lutein had a statistically significant effect on visual field and
this effect increased in the model assuming a 6-week delay in effect of lutein.
These results suggest that lutein supplementation improves visual
field and also might improve visual acuity slightly, although these results
should be interpreted cautiously. As a combined phase I and II clinical trial,
this study demonstrated the efficacy and safety of lutein supplementation.
DHA and fish oils
Invest Ophthalmol Vis Sci. 2015.
Docosahexaenoic Acid Slows Visual Field Progression in X-Linked Retinitis
Pigmentosa: Ancillary Outcomes of the DHAX Trial. Supplementation of DHA
significantly elevated blood DHA levels and reduced the rate of progression in
final dark-adapted thresholds and visual field sensitivity.
Further evaluation of docosahexaenoic acid in patients with retinitis pigmentosa
receiving vitamin A treatment: subgroup analyses.
Arch Ophthalmol. 2004. Berson EL, Rosner B, Sandberg MA, Weigel-DiFranco C, Moser A, Brockhurst RJ,
Hayes KC, Johnson CA, Anderson EJ, Gaudio AR, Willett WC, Schaefer EJ.
Berman-Gund Laboratory for the Study of Retinal Degenerations, Harvard Medical
School, 243 Charles Street, Boston, MA
To determine whether docosahexaenoic acid will slow the course of
retinal degeneration in subgroups of patients with retinitis pigmentosa who are
receiving vitamin A. A cohort of 208 patients with retinitis pigmentosa,
aged 18 to 55 years, were randomly assigned to 1200 mg of docosahexaenoic acid
plus 15 000 IU/d of vitamin A given as retinyl palmitate (DHA + A group) or
control fatty acid plus 15 000 IU/d of vitamin A (control + A group) and
followed up over 4 years. Seventy percent of the patients in each group were
taking vitamin A, 15 000 IU/d, prior to entry. We compared rates of decline in
ocular function in the DHA + A vs control + A groups among the subgroups defined
by use or nonuse of vitamin A prior to entry. We also determined whether decline
in ocular function was related to red blood cell phosphatidylethanolamine
docosahexaenoic acid level, dietary omega-3 fatty acid intake, or duration of
vitamin A use. Main outcome measures were Humphrey Field Analyzer visual field
sensitivity, 30-Hz electroretinogram amplitude, and visual acuity.
Among patients not taking vitamin A prior to entry, those in the DHA + A group
had a slower decline in field sensitivity and electroretinogram amplitude than
those in the control + A group over the first 2 years; these differences were not observed in years 3 and 4 of follow-up
or among patients taking vitamin A prior to entry. In the entire cohort, red
blood cell phosphatidylethanolamine docosahexaenoic acid level was inversely
related to rate of decline in total field sensitivity over 4 years. This was particularly evident over the first 2 years among those
not on vitamin A prior to entry. In the entire control + A group, dietary
omega-3 fatty acid intake was inversely related to loss of total field
sensitivity over 4 years.
The duration of vitamin A supplementation prior to entry was inversely related
to rate of decline in electroretinogram amplitude. For
patients with retinitis pigmentosa beginning vitamin A therapy, addition of
docosahexaenoic acid, 1200 mg/d, slowed the course of disease for 2 years. Among
patients on vitamin A for at least 2 years, a diet rich in omega-3 fatty acids
slowed the decline in visual field sensitivity.
Vitamin A and beta carotene
JAMA Ophthalmol. 2013. Treatment with 9-cis β-carotene-rich powder in
patients with retinitis pigmentosa: a randomized crossover trial. Treatment with
9-cis β-carotene significantly increased retinal function in patients with RP
under the tested conditions.
Cochrane Database Syst Rev. Dec 19 2013. Vitamin A and fish oils for retinitis pigmentosa. Based on the results of three RCTs, there is no clear evidence for benefit of treatment with vitamin A and/or DHA for people with RP, in terms of the mean change in visual field and ERG amplitudes at one year and the mean change in visual acuity at five years follow-up.
Studies in preterm and term human infants have suggested that a dietary supply of omega-3 fatty acids is essential for optimal visual development. Several basic science studies support the hypothesis that omega-3 fatty acids may be useful therapeutic agents for pathologies of the retina and lens. As part of a systematic review of the effect of omega-3 fatty acids on eye health, the purpose of this study was to conduct a systematic review of the scientific-medical literature to appraise and synthesize the evidence for the effects of omega-3 fatty acids in preventing the development or progression of retinitis pigmentosa. There are trends in improvement of some retinitis pigmentosa outcomes with omega-3 fatty acids in the higher quality studies. Clinical research is preliminary in this field, however. Accordingly, definitive answers will require significantly more observational and interventional clinical research. Can J Ophthalmol. 2006 Aug. The evidence for efficacy of omega-3 fatty acids in preventing or slowing the progression of retinitis pigmentosa: a systematic review. Department of Opthalmology, Ottawa Hospital Eye Institute, University of Ottawa, Ont., Canada.
Medical treatment
Curr Stem Cell Res Ther. 2015. Recent advances in treatment of retinitis
pigmentosa. Retinitis pigmentosa (RP) is a group of inherited retinal disorders
characterized by the progressive photoreceptors and pigment epithelial cells
dysfunction. It is the most common retinal degeneration, responsible for loss of
vision of most people worldwide. Until now its exact pathogenesis and etiology
are not clear. So far there is no approved therapy. New approaches for RP
therapy include cell transplantation, gene therapy, cytokine therapy, nutrition
therapy, and hyperbaric oxygen therapy. Such therapies for retinal degenerative
diseases are limited in their efficacy.